Korean Journal of Medicine : Vol. 59, No. 3, 2000 :,, 27 :,, : 6 56 thiazide 2,,., : : 120/60 mmhg, 70 /, 20 /, 36.2.,,. : 6,970/mm3, 13.2 gm/dl, 40.7

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1 : = A bs trac t= A cas e of pineal g erminoma pre s e nting w ith diabe te s ins ipidus Sam Seok Park, M.D., Young Keun Choi, M.D., Woo Hyung Bae, M.D., Seok Man Son, M.D., In Ju Kim, M.D. and Yong Ki Kim, M.D. Department of Internal M edicine, College of M edicine, Pusan N ational University, Pusan, K orea Central diabetes insipidus caused by pineal gland neoplasm is rare. Here, we describe a case of central diabetes insipidus and pineal germinoma with seeding to pituitary stalk along CSF pathway. A 27 year- old male patient was admitted due to polyuria, polydipsia, headache, vomiting, diplopia, and decreased visual acuity for recent two months. Urine osmolality measured after water deprivation was below 100mOsm/ kg. However, urine osmolality increasd above 300 mosm/kg with pitressin administration. Brain MRI showed a 2cm sized mass with seeding into pituitary stalk along CSF in pineal region. Surgical biopsy revealed pineal germinoma. T wo cycles of chemotherapy with etoposide and cis- platin were done followed by brain irradiation(2,500cgy). Follow up brain MRI after second chemotherapy showed complete remission. T he patient had no neurologic and endocrinologic deficit after the treatment.(korean J Med 59: , 2000) Key Words : Pinealoma; Germinoma; Diabetes insipidus, Neurogenic, (,, ), 1).,. (pinealomas) (ectopic pinealoma) 1% 2).,,, 3-8), 9). 1. : : :, 110, E- mail : psso911@hanmail.net

2 Korean Journal of Medicine : Vol. 59, No. 3, 2000 :,, 27 :,, : 6 56 thiazide 2,,., : : 120/60 mmhg, 70 /, 20 /, 36.2.,,. : 6,970/mm3, 13.2 gm/dl, 40.7%, 259,000/ mm3, 140 meq/l, 3.4 meq/l, BUN 12 mg/dl, creatinine 0.9 mg/dl. - human chorionic gonadotropin( - hcg)197 IU/ ml( 5 IU/mL), - hcg4 IU/mL, alpha fetoprotein(afp)4.4ng/ml( 12 ng/ml), AFP0.60 ng/ml, antidiuretic hormone(adh)1.8 pg/ml( 7.6 pg/ml). : 2 kg 300 mosm/kg. 5 50% (T able 1). 48 ng/ml(20 ng/ml) (T able 2). : 222cm mm, (Figure 1). Figure 1. Gadolinium enhanced brain MRI(sagittal image) shows a well- enhancing round soft tissue mass(22 2cm) in pineal region. The mass extends into cerebral aqueduct and causes the dilatation of lateral and third ventricles. A 810mm sized mass is also noted at the pituitary stalk. Table 1. Water deprivation and pitressin test Hr(s) Serum osmolality(mosm/ kg) Urine osmolality(mosm/ kg) pitressin 5U SC T able 2. Basal hormone study GH(ng/mL) Prolactin(ng/ ml) LH(mIU/mL) FSH(mIU/mL) T estosterone(ng/dl) Cortisol(/dL) 07: 00 08: 00 09:

3 Sam Seok Park, et al : A case of pineal germinoma presenting with diabetes insipidus.. Figrue 2. Germinoma with a multinodular pattern produced by fibrous trabeculae containing a lymphoid infiltrate. Germinoma cells have clear to light eosinophilic cytoplasm and nuclei with prominent, central nucleoli. Lymphoid infiltration is intense in and around the fibrous trabeculae of neoplasm, but also seen intermingled with the neoplastic cells(h&e, 100). Figure 3. Follow- up brain MRI(sagittal image) shows no lesion of pineal area and hydrocephalus. : thiazide, DDAVP, (occipital transtentorial approach) (Figure 2). EP regimen(etoposidecis- platin) 2, 2 (Figure 3). 3 2,500 cgy. 6,,,, 10).,, (primary polydipsia),.,, 2).,,. 3,., 30. 2), 3. 80% 11).,,,. 9%, 9% 50%, 50% 1). 2 kg 300 mosm/kg. 5 50%

4 : (isolated enlargement of pituitary stalk) 12). 12). 13),, Niznikiewicz 14)310, Pomarede 15) 13Oka 16)9. - HCG, AFP 5%, 30%2). - HCG., 2), 712% 8).,, 8).,,. 8), 17)., 17). 2 18,19)., 20), 6..,. 1. RE F E R E N C E S 1) Fauci AS, Braunwald E, Isselbacher KJ, Wilson JD, Martin JB, Kasper DL, Hauser SL, Longo DL. Harrison's principles of internal medicine. 14th ed. p. 2004, N ew W ork, M cgraw- Hill Companies, Inc, ) Wilson JD, Foster DW, Kronenberg HM, Largen PR. W illiams textbook of Endocrinology. 9th ed. p. 359, Philadelphia, W.B, Saunders, ) Tarng- DC, Huang- T P. Diabetes insipidus as an early sign of pineal tumor. A m J N ephrol 15: , ) Adam P. Evaluation and management of diabetes insipidus. A m Fam Physician 55: , ) Prahlow, Joseph A. N eoplasms of the pineal region. South M ed J 89: , ) Hill P, Lepre F, A llison R. CN S germinomas : curable tumours in two adolescents. J Paediatr Child Health 30(1):68-71, ) Axelrod L. Endocrine dysfunction in patients with tumors of the pineal region. Pineal T umors. p.61, N ew York, M asson, ) J. Randy. Jinkins, Claudia da Costa Leite. N eurodiagnostic Imaging: pattern analysis and differential diagnosis. p. 562, philadelphia, Lippincott- Raven, ),,,,,, (5): , ) Miller KL. Diabetes insipidus. AN N A J 23(3): , ) Portfolio ML, de Gonzalez CM, Sangines JB, Zapata GS. Pineal region tumors. Int Surg 67: ,

5 5: 1 12) Wilkins RH, Roengachary SS. N eurosurgery. 2nd ED. p.1012, M cgraw Hill, ) Tarng DC, Huang TP. Diabetes insipidus as an early sign of pineal tumor. Am J N ephrol 15: , ) Niznikiewicz J. Diabetes insipidus as a preliminary sign of brain tumor. N eurol N eurochir Pol 18: , ) Pomarede R, Finidori J, Czernichow P. Germinoma in a boy with precocious puberty. Evidence of hcg secretion by the tumor cells. Child's Brain 11: , ) Devita VT, Hellman S, Rosenberg SA. Cancer principle and practice of oncology. 5th ed. p.2064, Philadelphia, Lippincott- Raven, ) Patel SR, Buckner JC, Smithson WA, Scherthauer BW, Groover RV. Cisplatin- based chemotherapy in primary central nervous system germ cell tumors. J N eurooncol 12:47-52, ) Itoyama Y, Kochi M, Yamashiro S, Yoshizato K, Kuratsu J, Ushio Y. Combination chemotherapy with cisplatin and etopiside for hematogenous spinal metastasis of intracranial germinoma. N eurol M ed chir 33(1):28-31, ) Kobayashi T, Yoshida J, Ishiyama J, Noda S, Kito A, Kida Y. Combination chemotherapy with cisplatin and etoposide for malignant intracranial germ - cell tumors. An experimental and clinical study. J N eurosurg 70(5): , ) Rubin P, Kramer S. Ectopic Pinealoma. A radiocurable neuroendocrinologic entity. Radiology 85(3): ,

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